Saturday, June 30, 2012

Yesterday, I had the opportunity to participate in a Quality Improvement project with the CF clinic. There had been a kick off meeting to this initiative about a month ago and it was decided that both education and technology were of equal importance and that there was an overlap in using technology to educate. The goal is to improve outcomes for CF patients through more targeted education. So for example, the doctors in our clinic have a pre-clinic meeting where they discuss the patients that will be seen in the upcoming week and decide who needs to see them (doc, dietician, RT, etc). The parents felt that it was important to be involved in this meeting, in some capacity, so that the doctors and nurses and whoever else could know in advance of a visit whats been going on with the patient and what the patient and/or parent feel its important to discuss. This could lead to more effective visits. We also talked about how to identify when doctors need to bring up certain topics with patients - everything from checking to make sure their vest is fitting properly and working the way its supposed to, to things like the fact that CF kids aren't supposed to be in hot tubs. There is so much information to be known and there is a fine balance between giving people what they need and bombarding them with information. Enter C3N.

There's already something being developed that is going to change health care for people chronic conditions and its seriously awesome. C3N stands for Collaborative Chronic Care Network. This is how their website describes exactly what it is:

The Collaborative Chronic Care Network (C3N) Project is an innovation lab, where patients, clinicians and researchers work together as co-designers in a learning, social production system for health that aims to transform the system of chronic illness care. Working with ImproveCareNow, we are creating the first C3N for patients with IBD. By combining large data registries and making them accessible and interactive, the C3N Project drives action and innovation to create a more reliable and accountable care delivery system for children and their families dealing with chronic gastrointestinal diseases. And with an open-source framework, this project is developing a means to overcome barriers that involve concerns about intellectual property, data sharing and privacy, and medicolegal1 liability. The C3N Project is the future of chronic illness care. And it is already in play.

While its being created to help all chronic conditions, it was piloted for Crohn's disease. The meeting I had yesterday at Children's was about bringing it to the CF world. Here is a short video on how it is going to work. Its about 5 minutes long but stick with it because it really explains nicely how the project works. And just to be clear, this is all still in the works.

Isn't that awesome!? Can't you just see the possibility for use in the CF world? One of the things we discussed yesterday was the fact that you don't know what you don't know. Sometimes you don't know what questions to ask or what you want or need to know at different points in caring for your sick child. The C3N project can gather data on what people are doing or are concerned with or are talking about at certain different points and share that knowledge with others. Doctors can target their care plan based on what patients themselves show what is important.

There has been some press about this project too. Check out these articles if you have time because they are very interesting. Nothing like this has ever been done, but its really different and exciting and might just be exactly what we need.

I think one of the things I like most about this is the ability to improve outcomes for patients with chronic condition simply by streamlining data and information and opening the lines of communication between patients and clinicians. I'm very excited to be working on this project with the CF clinic and and only imagine how this will change the world for people with CF!

Sunday, June 24, 2012

Summer is in full swing here in our house. After we pull ourselves together in the morning, we spend out days running around outside, playing in the pool, going to parks, playing with friends, and having all sorts and kinds of fun. I can't complain to much about how things have been going. Drew is halfway through his Cipro treatment. I've been reading some CF message boards about how people deal with pseudomonas that isn't the aeruginosa strain. I'm getting some mixed messages, where some peoples clinics only get concerned about pseudomonas aeruginosa and don't even treat the other pseudomonas strains, and others treat all strains of pseudomonas the same way, with Tobi and oral antibiotics or IV antibiotics. Since I don't have any experience with anything other than aeruginosa I don't know what the best course of action is. I will say that having Drew on antibiotics right now is the best thing for him, as Ella and I both have a nasty cough and lung infection. We are on antibiotics and hoping that the remaining 3 don't catch anything. I think that the fact that we've got Drew on antibiotics is kind of protecting him from this and I am very very grateful for that.

Drew has started to show the slightest level of protest against taking his enzymes. I really think its a control thing. He is forced to do his breathing treatments and airway clearance so many times a day and he has no choice when it comes to going to the doctor and having tests done all of the time. So the things in his life that he has control over, like eating for example, and now taking his meds, he seems to be fighting back. Again, its just ever so slightly. He doesn't fight us on any of the stuff he has to go through every day, month after month. He is such a good kid with all that he has to go through ,but I don't know what you do when he needs this medicine to eat and to survive and to thrive and he doesn't want to take it anymore. 720. That's how many pills Drew takes in a month. I'd probably protest too if I had to swallow 720 pills every single month, 24 pills every day. Think about that next time you think you're taking a lot of medicine!

We are heading out to Philly in just over a week and I couldn't be less ready. I haven't even thought about packing and haven't had moment to think about the logistics of the actual trip. I'd love to drive through the night when they are all asleep and not screaming at me and at eachother because someone touched someone or having to watch Toy Story is the most horrible thing in the world. Between feedings and diaper changes and potty breaks and movie requests and sippy cup refills I suspect it will be a long trip. I should give them a little credit as last year we drove all the way to Charleston and only made 1 stop the whole way. We also did not have a 3mo old with us. Now that I'm typing about it I'm starting to think of all the things I need to pack and where and how.

It will be so nice to be someplace else for a couple of weeks. While the packing and the travel and the disruption in schedules may be a pain, having someone else there to help me during the day will be a godsend. Equally as exciting (for me, not you) is that Martin will be here remodeling our family room that I have been dying to have remodeled since we moved in 3 years ago. I cannot wait for the wood paneling to disappear!

I don't know that I'll have much time to blog before we take off next week. I really hope that all goes smoothly health wise. This will be the longest we are away from our clinic. Drew's health has generally been pretty good, but I won't be able to run down to the hospital for a quick check up if he catches something or isn't acting quite right. We do have CHOP not to far away and if he needs to be see our doctors can contact the doctors at that CF clinic. But I hope we can just go away for a couple of weeks and relax and have fun!

Saturday, June 9, 2012

So it turns out that we have a different form of pseudomonas than CF people normally get. The common pseudomonas is aeruginosa and the strain that Drew has is called fluorescens.Its not a good or bad thing, its just a different kind. So the good aspect is that the pseudomonas aeruginosa that we eradicated last September is still gone! We are going to take the same approach and try to eradicate this pseudomonas now too. The doctors are still concerned that with the condition of his airways, Tobi wouldn't be our best option. You may recall from our past uses of it Drew's terrible reactions that nearly landed us in the hospital. The other inhaled option, Cayston, caused similar breathing difficulties. Even though its been almost a year, they think its best to wait a little longer to try those again as we don't want to make things worse. In the past we have used IV Ceftazadime and Tobramycin and been able to get rid of it. But this time we are going to approach things differently. We are going to try just using Cipro orally for 30 days. One common treatment plan for pseudomonas is inhaled Tobi for 28 days followed by Cipro for 30 days, and the doctor told us that recent research has shown no real difference in people who did just one or the other vs both. So since we don't use Tobi right now, we are going to see what Cipro can do for us. He's been on it for 1 day and already puked once. Its just so strong it upsets his stomach. And it causes a terrible sun sensitivity so we need to keep the boy covered up when we are outside...which is all the time. Oh well. The antibiotics will end just before we leave for our trip back east, and once we return we will re-culture him to see if we were able to get rid of it. If its not gone, then we will go to IV to try to get rid of it and hopefully succeed. So there is a plan in place and I'm happy with it. Hopefully things remain under control and we can enjoy our trip!

Friday, June 8, 2012

Stupid, unpredictable CF. We just got back the culture results from our clinic visit on Monday and it grew Pseudomonas again. I'm waiting for the nurse to call me back with a treatment plan. In the past, we have been able to eradicate it with a PICC line and IV antibiotics. But now that Drew is a little bigger I'm slightly more inclined to try the inhaled antibiotics that are normally used to treat pseudomonas infections. The problem is that he has had such a bad reaction to some of the inhaled drugs in the past. He's bigger now and his airways are stronger so I kind of want to try it. But if we try that for 30 days and the pseudomonas isn't gone and we need a PICC line then that cuts into our summer trip to PA and ruins things for everyone. So part of me wants to start with the PICC that we've had success with in the past, but I know that that could be far less pleasant for Drew even if it means a shorter treatment time. This is one of the things I hate about CF, its ability to ruin a good day with the snap of a finger, and to turn the world upside down for our family. I'm still waiting to hear back with what the doctor thinks is best. She usually goes with what I say I want, but I don't know what I want. I don't know what I think is best. It would be great if we can do the inhaled stuff and he tolerates it fine and the pseudomonas goes away. Bit if we do a 30 day treatment of that, we are scheduled to be in PA when it finishes, so how can we even get him re-cultured to see if its gone? And if it isn't gone, I can't get a PICC in PA and have homecare deliver meds to my parents house. Ughh. Bad end to a good week. Stupid CF.

Tuesday, June 5, 2012

I found this online tonight and it touched my heart. I wish I could share this with everyone I talk to in the NICU every other week. Its a little bit long but its so worth the read.

What I Would Tell YouI sensed someone watching me as I comforted my daughter after a particularly traumatizing dentist appointment at the Children’s Hospital. I looked up and saw you staring at us from across the waiting lounge. I didn’t pay much attention, as I have grown accustomed to the curious eyes of onlookers. Our daughter was born 7 ½ years ago and after an abrupt lack of oxygen at birth, she changed the course of our lives forever. Perhaps, our lives unfolded exactly as they were meant to — they just didn’t unfold in the way we had imagined or planned.I talked to my daughter, kissed her and hugged her. I was giving her a brief break before putting her through the next traumatic experience of the day ~ the car ride home. Having cerebral palsy is the least of her worries but this condition can turn a car seat into a torture chamber.I stood up to gather our things, my daughter in my arms, and it was then that I noticed you were holding an infant. It was difficult to know for certain how old she was. I knew immediately, though, that you were one of us. I knew that only recently your life had changed drastically and you sat here in this Children’s Hospital wondering, “How did we get here?” I should have recognized that shocked stare because I once had it, too. And I assume that the man sitting next to you, looking equally tired and shocked, was your husband.I made my way toward the doors and as I passed you, our eyes met and I smiled at you. You smiled back and for a moment I knew that you knew that I understood.If I could, I would tell you although you might not believe it right now, you will be okay. I would tell you to dig deep within yourself because you will find the strength and resilience somehow and it will surprise you. I would tell you to honour your feelings and let the tears flow when they need to. You will need the energy for more important things than holding in emotions.I would tell you that the man sitting next to you might cope differently and he might even want to run the other way. But I would tell you to hang on because he is scared and he really doesn’t want to leave you. I would tell you to look after yourself so that you can care for your daughter. Don’t underestimate the power of good nutrition, exercise, sleep, supplements and an empathetic therapist.I would tell you that grief will come and it will confuse you because how can something that brings such joy also bring such sadness? I would tell you to let people into your lives to help you. Our children really do require a village to raise them. Access all of the services and resources available. Find someone who can learn how to care for your child so that you can have breaks and so you and your partner can go on dates… even little ones like a twenty minute stroll outside holding hands, sharing wine on the deck or even catching a movie.I would tell you that you know your child best of all and no matter what you are told by the doctors and other professionals who will be a part of your life, YOU know the answers. You will teach them about your child. At times you will question the validity of your intuition but after a while you will become profoundly aware of how accurate your gut feelings are when it comes to your child.I would tell you not to be a martyr. Caring for your child will require tremendous focus and unimaginable energy and it can burn you out and make you sick when you least expect it. I would tell you to let your guard down along the way so that you can stay healthy in your mind and spirit.I would tell you to seek out other mothers like yourself. This is, indeed, the road less travelled and you will feel very alone along the way especially in the company of healthy children and their parents. Yes, you will feel very isolated but know that we are here. Sometimes you have to look a little harder but we are here. You can find us online, in support groups and wandering the halls of the Children’s Hospital.I would tell you that you will know far too much about the human anatomy, neurology, gastro-enterology, feeding tubes, pharmaceuticals, and so on, than a mother should ever have to know. I would also tell you to do some research to inform yourself but be very careful not to be overwhelmed by the internet and all of the information available to you. Having some trust in what your child’s specialists tell you can be very grounding. Other mothers and fathers of children like ours can be a wealth of information.I would tell you that this isn’t an easy life. It is tough: there is no doubt about it but you are very capable and the rewards are great. You may never see your child graduate from university, walk down the aisle or give birth to your grandchildren but you will feel pure joy when your child laughs for the first time at the age of 3 years and 8 months. You will celebrate the moment when you connect with your non-verbal child. You will call your spouse at work to tell him that she has gained 4oz. because weight gain is always a struggle with our children.I would tell you that you will have to witness procedures and surgeries and suffering well beyond what any parent should ever have to bear. But, I would tell you that you will be courageous and comforting because your child will be experiencing far more suffering than any child should ever have to endure.I would tell you that your life will not resemble the life you had planned. It will be as though you landed in Holland instead of Italy but after some time, you will adjust the dreams you had and this reality will be normal to you. You will dream new dreams.I would tell you that you might find yourself staring death in the face during close calls. You will be asked to fill out DNR (Do Not Resuscitate) forms and although you might make decisions to not resuscitate in the event of a cardiac arrest, when the moment arises, you will panic to think that it could all come to an end. And I would tell you to not feel guilty in the darkest moments when you pray to God to take your child if it would mean the suffering would end. This might horrify you but know that your love for your child is so great that at times you will believe that death would be a blessing.I would tell you that others will not get it. They can’t. This is a very unique and complex journey on all levels. We cannot expect anyone to get it. And I would tell you that people — the cashier at the grocery store or your insurance broker or even your hair stylist — will say stupid things like, “God only gives these special kids to special mothers” and “God will only give you what you can handle.” You will nod and smile but eventually you will look them right in the face and tell them that those simple maxims are a bunch of bullshit.I would tell you that imagining your future will be bittersweet and may involve a Plan A and a Plan B. Plan A will be what you will do if your child outlives the predicted life expectancy set forth by the experts and Plan B will come into play if they do not. You will catch yourself casually discussing your future with the code phrases of Plan A and Plan B.I would tell you that grief will creep up on you after years have passed and you least expect it like at a wedding when the father and bride have their first dance or when you hear a birth announcement. It will also creep up on you when you see yourself in a new mother who is just beginning this journey.I would tell you that you will recognize her because she is you from 7 ½ years ago. And you will want to run to her and hug her and tell her that everything will be okay. You will want to save her from the pain and the hardship and the unknown.But I would tell you that when you find yourself sitting at the Children’s Hospital and you see a new mom and dad who are just starting this journey, you smile at them and walk by as they have their own path to travel and it will be different than yours. It may be longer or shorter. It may be more or less complicated.I would tell you that her searching eyes are looking for some sign that she will survive this. And you, smiling as you pass, with your child arching all over your shoulder, will let her know that yes, she will survive this and may even thrive.Julie KeonJune 29th 2011

Things around here are just plain wild these days. I wake up early and go to bed late wondering where the day went. I have the best intentions for getting things done, both with the kids and for the kids and around the house and otherwise, but there just never seems to be enough time. I sit for 5 minutes and I'm 5 minutes behind schedule. I miss being able to sleep in on the weekends and veg out on the couch for a couple of hours at night.

We are on behavior renovation 2012 around here. After Jake came along, schedules got screwy and attentions got shifted and before we knew it everyone was yelling at everyone, fuses were short, fighting was common and time outs were plentiful. We were losing and no one was happy. But, now that Jake is 10 weeks old (or maybe 11? I honestly lost count, poor #4) I'm on a mission to regain control and respect around here and I have to say that after just a week, things are starting to improve. All they've needed is some structure, some consistent discipline, and some individual attention. It means we haven't left the house in a while, but that's okay since it seems to be working. We still have some work to do, but hopefully we continue down the path to change and happiness for all.

Drew had a clinic visit on Monday, so we are in the middle of the dreaded wait for culture results. They should be back on Friday. He looks and sounds good so fingers are crossed that we come back clean.

Funny story about the clinic visit. Last time we were there, his weight was down ever so slightly. So in the past 3 months we have been working to keep him beefed up and gaining. No one was concerned with his weight, just concerned that it might start to trend downward. So we started adding half and half to his milk and making him extra buttery waffles loaded with peanut butter for breakfast and other things like that. We got recipes for super high calorie breakfast sandwiches that most people would dream about eating but that are only good for CFers. It was a little excessive, but Drew has always had great weight, being in the 85th percentile for weight, and we have never had to try to beef up his calorie/fat intake. For whatever reason, he doesn't seem to have the same caloric needs that a typical CF kid does, right now anyway. He got sick about 3 weeks ago and ended up losing a pound, but again it wasn't anything to worrying. So yesterday I go to the doc and he hops on the scale and it says 11.3kg or 24.9lbs. That is down almost 5 pounds from the last time! And when you only weigh 30lbs, thats a sixth of your body weight! My jaw hit the floor. I thought we were in big trouble. They weighed him again and got the same number. So I talked to the nurse who suggested getting back in touch with the food psychologist and I talked to the doctor for a while and we talked to the respiratory therapist all giving ideas and suggestions for things we can do to try to turn this around. This weight put him in the 14th percentile and I was getting the scoop on what we need to do and when we should be concerned enough to discuss a g-tube and all of that fun stuff. Then the dietician came in. And she just couldn't buy that he was down that much, based on the way he looked and everything else. She took him back to the scale and weighed him again and he was at 14.3kg. Both of us were puzzled, so we took him to a different scale - same reading, 14.3kg or 31.5lbs! He was actually UP in weight! So that stress filled two hours of talking and worrying and planning were all for naught. I watched the first nurse weight him those first two times and it did say 11.3kg, so I'm not sure what exactly was going on but I sure am glad we got it sorted out! So overall we got two thumbs up and were instructed to continue doing what we are doing because he looks and sounds great right now. I'm one happy mama!

"65 Roses"is what some children with cystic fibrosis call their disease because the words are much easier for them to pronounce.

This blog, 66 Roses, is dedicated to finding the cure.

Cystic fibrosis is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). Thick mucus clogs the lungs and leads to life threatening infection. The pancreas is also obstructed by this thick mucus production, imparing digestion and leading to malnutrition. - www.cff.org